A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice
A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice
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DOI：
10.1172/jci200421055
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发表时间：
2004-10-01
影响因子：
15.9
通讯作者：
Murray, JA
中科院分区：
文献类型：
--
作者：
Marietta, E;Black, K;Murray, JA
疱疹样皮炎（DH）是一种自身免疫性起泡性皮肤病，与麸质敏感性有关。它表现为丘疹性水疱性皮疹，通常与肠病有关。当患者接受无麸质饮食和/或氨苯砜时，皮疹消退。DH以及乳糜泻与DQ 2和DQ 8密切相关。描述了一种新的DH小鼠模型，其利用NOD背景和HLA-DQ 8转基因。DQ 8的添加有助于对麦醇溶蛋白的敏感性，并且NOD背景的添加有助于自身免疫和发病机制。90只NOD DQ 8（+）小鼠中有15只对麸质过敏，出现了与DH相似的水疱病理。观察到真皮的中性粒细胞浸润、真皮-表皮交界处的伊加沉积以及使用含或不含氨苯砜的无麸质饮食的起泡现象的完全逆转。检查的3只起泡小鼠均未出现小肠病理学。这种DH动物模型将是有用的。以确定伊加沉积物的特异性，以及由于麸质摄入而在皮肤中发生的致病机制。
Dermatitis herpetiformis (DH) is an autoimmune blistering skin disorder that is associated with gluten sensitivity. It presents as a papulovesicular rash and is often associated with enteropathy. The rash resolves when the patient is placed on a gluten-free diet and/or dapsone. DH, as well as celiac disease, is tightly associated with DQ2 and DQ8. A novel mouse model for DH is described that utilizes the NOD background and the HLA-DQ8 transgene. The addition of DQ8 contributes sensitivity to gliadin, and the addition of the NOD background contributes to autoimmunity and pathogenesis. Fifteen NOD DQ8(+) mice of 90 that were sensitized to gluten developed blistering pathology similar to that seen in DH. Neutrophil infiltration of the dermis, deposition of IgA at the dermal-epidermal junction, and a complete reversal of the blistering phenomenon with the administration of a gluten-free diet with or without dapsone were observed. None of the 3 blistering mice examined had small-bowel pathology. This animal model of DH will be useful. to determine the specificity of the IgA deposits, as well as the pathogenic mechanisms that occur in the skin as a result of gluten ingestion.